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      Cystic Fibrosis-Related Diabetes Mellitus and Pregnancy: A Retrospective Study

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          Abstract

          Introduction

          Cystic fibrosis-related diabetes mellitus (CFRDM) is becoming a more common issue in pregnancy care as the life expectancy of females living with cystic fibrosis has improved, with an increasing number of pregnancies in this population. Despite the Republic of Ireland having the highest incidence of cystic fibrosis globally, there is limited Irish data on pregnancy outcomes for those with CFRDM. This study aimed to retrospectively review maternal and foetal outcomes of pregnancies affected by maternal CFRDM.

          Methods

          The patient records of all women with CFRDM who attended the National Maternity Hospital Dublin for obstetric care between 2015 and 2019 were retrospectively reviewed.

          Results

          A search of patient records identified 15 pregnancies in 12 women with CFRDM during the study period. CFRDM was diagnosed pre-conception in ten of the 15 pregnancies. Median neonatal weight at birth was lower in women with CFRDM diagnosed pre-conception compared to women diagnosed during pregnancy (2.8 vs. 3.02 kg). The median weight gain in women with CFRDM diagnosed pre-conception was 10.9 kg compared to 11.9 kg for those diagnosed during pregnancy. The majority of women (62.5%) with CFRDM diagnosed pre-conception delivered via caesarean section. Admission for CF exacerbations during pregnancy in women with CFRDM diagnosed pre-conception was very common (87.5%) compared with 75% of those diagnosed during their pregnancy.

          Conclusion

          Women diagnosed with CFRDM were likely to require caesarean section, to be treated with insulin, and to be frequently admitted to hospital for CF exacerbations. Our review highlights the importance of good glucose control, stable cystic fibrosis before pregnancy and a multidisciplinary team approach.

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          Most cited references20

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          Cystic fibrosis.

          J. Elborn (2016)
          Cystic fibrosis is a common life-limiting autosomal recessive genetic disorder, with highest prevalence in Europe, North America, and Australia. The disease is caused by mutation of a gene that encodes a chloride-conducting transmembrane channel called the cystic fibrosis transmembrane conductance regulator (CFTR), which regulates anion transport and mucociliary clearance in the airways. Functional failure of CFTR results in mucus retention and chronic infection and subsequently in local airway inflammation that is harmful to the lungs. CFTR dysfunction mainly affects epithelial cells, although there is evidence of a role in immune cells. Cystic fibrosis affects several body systems, and morbidity and mortality is mostly caused by bronchiectasis, small airways obstruction, and progressive respiratory impairment. Important comorbidities caused by epithelial cell dysfunction occur in the pancreas (malabsorption), liver (biliary cirrhosis), sweat glands (heat shock), and vas deferens (infertility). The development and delivery of drugs that improve the clearance of mucus from the lungs and treat the consequent infection, in combination with correction of pancreatic insufficiency and undernutrition by multidisciplinary teams, have resulted in remarkable improvements in quality of life and clinical outcomes in patients with cystic fibrosis, with median life expectancy now older than 40 years. Innovative and transformational therapies that target the basic defect in cystic fibrosis have recently been developed and are effective in improving lung function and reducing pulmonary exacerbations. Further small molecule and gene-based therapies are being developed to restore CFTR function; these therapies promise to be disease modifying and to improve the lives of people with cystic fibrosis.
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            Effect of treatment of gestational diabetes mellitus on pregnancy outcomes.

            We conducted a randomized clinical trial to determine whether treatment of women with gestational diabetes mellitus reduced the risk of perinatal complications. We randomly assigned women between 24 and 34 weeks' gestation who had gestational diabetes to receive dietary advice, blood glucose monitoring, and insulin therapy as needed (the intervention group) or routine care. Primary outcomes included serious perinatal complications (defined as death, shoulder dystocia, bone fracture, and nerve palsy), admission to the neonatal nursery, jaundice requiring phototherapy, induction of labor, cesarean birth, and maternal anxiety, depression, and health status. The rate of serious perinatal complications was significantly lower among the infants of the 490 women in the intervention group than among the infants of the 510 women in the routine-care group (1 percent vs. 4 percent; relative risk adjusted for maternal age, race or ethnic group, and parity, 0.33; 95 percent confidence interval, 0.14 to 0.75; P=0.01). However, more infants of women in the intervention group were admitted to the neonatal nursery (71 percent vs. 61 percent; adjusted relative risk, 1.13; 95 percent confidence interval, 1.03 to 1.23; P=0.01). Women in the intervention group had a higher rate of induction of labor than the women in the routine-care group (39 percent vs. 29 percent; adjusted relative risk, 1.36; 95 percent confidence interval, 1.15 to 1.62; P<0.001), although the rates of cesarean delivery were similar (31 percent and 32 percent, respectively; adjusted relative risk, 0.97; 95 percent confidence interval, 0.81 to 1.16; P=0.73). At three months post partum, data on the women's mood and quality of life, available for 573 women, revealed lower rates of depression and higher scores, consistent with improved health status, in the intervention group. Treatment of gestational diabetes reduces serious perinatal morbidity and may also improve the woman's health-related quality of life.
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              Cystic Fibrosis–Related Diabetes: Current Trends in Prevalence, Incidence, and Mortality

              OBJECTIVE Cystic fibrosis (CF)-related diabetes (CFRD) diagnosis and management have considerably changed since diabetes was first shown to be associated with a poor prognosis in subjects with CF. Current trends in CFRD prevalence, incidence, and mortality were determined from a comprehensive clinical database. RESEARCH DESIGN AND METHODS Data were reviewed from 872 CF patients followed at the University of Minnesota during three consecutive intervals: 1992–1997, 1998–2002, and 2003–2008. RESULTS CFRD is currently present in 2% of children, 19% of adolescents, and 40–50% of adults. Incidence and prevalence are higher in female subjects aged 30–39 years; otherwise, there are no sex differences. In younger individuals, CFRD without fasting hyperglycemia predominates, but fasting hyperglycemia prevalence rises with age. CFRD mortality has significantly decreased over time. From 1992–1997 to 2003–2008, mortality rate in female subjects dropped by >50% from 6.9 to 3.2 deaths per 100 patient-years and in male subjects from 6.5 to 3.8 deaths per 100 patient-years. There is no longer a sex difference in mortality. Diabetes was previously diagnosed as a perimorbid event in nearly 20% of patients, but of 61 patients diagnosed with diabetes during 2003–2008, only 2 died. Lung function but not nutritional status is still worse in CF patients with diabetes compared with those without diabetes. Nutritional status and pulmonary status are similar between patients without fasting hyperglycemia and those with fasting hyperglycemia. CONCLUSIONS Previously noted sex differences in mortality have disappeared, and the gap in mortality between CF patients with and without diabetes has considerably narrowed. We believe that early diagnosis and aggressive treatment have played a major role in improving survival in these patients.
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                Author and article information

                Contributors
                mhatunic@mater.ie
                Journal
                Diabetes Ther
                Diabetes Ther
                Diabetes Therapy
                Springer Healthcare (Cheshire )
                1869-6953
                1869-6961
                21 February 2022
                21 February 2022
                March 2022
                : 13
                : 3
                : 481-487
                Affiliations
                [1 ]GRID grid.411596.e, ISNI 0000 0004 0488 8430, Endocrinology Department, , Mater Misericordiae University Hospital, ; 30 Eccles Street, Dublin 7, D07XA09 Ireland
                [2 ]GRID grid.415614.3, ISNI 0000 0004 0617 7309, The National Maternity Hospital, ; Holles Street, Dublin, Ireland
                [3 ]GRID grid.7886.1, ISNI 0000 0001 0768 2743, University College Dublin, ; Dublin, Ireland
                Author information
                http://orcid.org/0000-0002-9693-1226
                Article
                1223
                10.1007/s13300-022-01223-1
                8934781
                35190969
                c10da89b-a490-4fa1-a7b7-fd783811819f
                © The Author(s) 2022

                Open AccessThis article is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License, which permits any non-commercial use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc/4.0/.

                History
                : 18 November 2021
                : 2 February 2022
                Categories
                Original Research
                Custom metadata
                © The Author(s) 2022

                Endocrinology & Diabetes
                cystic fibrosis and pregnancy,cystic fibrosis-related diabetes,cfrdm,diabetes in pregnancy

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